Papillary renal cell carcinoma

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Papillary renal cell carcinoma, abbreviated PRCC, PaRCC and papillary RCC, is the second most common type of renal cell carcinoma.

Papillary renal cell carcinoma
Diagnosis in short

Papillary renal cell carcinoma. H&E stain.
LM cuboidal or low columnar cells (simple or pseudostratified) on papillae, interstitial foam cells in the vascular cores
Subtypes no World Health Organization recognized subtypes; defunct subtypes: "type 1" and "type 2", oncocytic variant
LM DDx clear cell renal cell carcinoma, clear cell papillary renal cell carcinoma, metanephric adenoma (esp. solid PaRCC type 1), collecting duct carcinoma (esp. PaRCC type 2), renal papillary adenoma, acquired cystic disease-associated renal cell carcinoma, urothelial carcinoma, renal mucinous tubular and spindle cell carcinoma, ALK translocation renal cell carcinoma, Xp11 translocation renal cell carcinoma
Molecular MET mutation (hereditary papillary renal cell carcinoma), FH mutation (hereditary leiomyomatosis and renal cell cancer)
Gross may be multifocal, must be >1.5 cm (if low ISUP grade), often necrotic-appearing (brown, soft, friable)
Grossing notes total nephrectomy for tumour grossing, partial nephrectomy grossing
Staging kidney cancer staging
Site kidney - see kidney tumours
Associated Dx acquired renal cystic disease (end-stage renal disease)
Syndromes hereditary papillary renal cell carcinoma, hereditary leiomyomatosis and renal cell cancer
Prevalence relatively common
Clin. DDx other kidney tumours
Treatment surgical excision, ablation

General

  • Historically, PaRCC was subclassified[1] into type 1 and type 2.
    • Type 1 and Type 2 are different on a cytogenetic and molecular basis.[2]

Epidemiology

Gross

  • Renal cortical mass usually >1.5 cm.
  • May be multifocal. ‡
  • Often necrotic appearing - light-to-dark brown, soft/mushy, friable.

Note:

  • ‡ Approximately 10% of PaRCCs are multifocal (based on a set of 5378 patients);[6] it is the renal tumour that is most commonly multifocal.

Microscopic

Features:[7]

  • Cuboidal or low columnar cell in papillae.
  • Interstitial foam cells in vascular cores - key feature.
    • Most sensitive and specific feature of PaRCC.[8]
  • Highly vascular.

Size criterion:

Mnemonic HIP: highly vascular, interstitial foam cells, papillae.

Note:

DDx:

Images

  • PaRCC - intermed. mag. (WC/Nephron)

  • PaRCC - high mag. (WC/Nephron)

  • PaRCC - very high mag. (WC/Nephron)

  • PaRCC - intermed. mag. (WC)

  • PaRCC - high mag. (WC)

  • PaRCC - very high mag. (WC)

  • PaRCC - high mag. (WC/Nephron)

  • PaRCC - very high mag. (WC/Nephron)

Histological subtyping

Historically, PaRCC was subtyped:[1][14]

  • Type 1 - single layer of cells on basement membrane - most important.
    • Usually low grade nuclear features, i.e. low ISUP nucleolar grade.
    • Other characteristics:
      • Clear cytoplasm.
      • Foamy macrophages - common.
      • Cells smaller.
  • Type 2 - pseudostratification of cells - most important.
    • Usually high grade nuclear features, i.e. high ISUP nucleolar grade.
    • Other characteristics:
      • Eosinophilic cytoplasm.
      • Foamy macrophages - uncommon.
      • Cells larger.

The WHO GU Blue Book 5th Ed. recommends against subtyping.

Another subtype:

  • Oncocytic - oncocytic cytoplasm.
    • Extremely rare ~ a few dozen reported.[15][16]
      • One "large" series was 14 cases.[17]

IHC

Features:[1]

  • AMACR +ve.
  • HMWCK (34betaE12) +ve.
  • Panker (AE1/AE3) +ve.
  • CK7 +ve ~90% of type 1, 20% of type 2.
  • CD10 +ve.[18]

Others:

  • GATA3 +ve - oncocytic variant of PaRCC; negative in other PaRCC subtypes.[19]

Type 1 versus Type 2:[20]

  • CK7:
    • Type 1 ~ 100%.
    • Type 2 ~ 19%.
  • CK19:
    • Type 1 ~ 100%.
    • Type 2 ~ 53%.

Metanephric adenoma vs. PaRCC type 1:[21]

  • AMACR +ve.
  • WT-1 -ve.
  • CD57 -ve.

Molecular

Features:[22]

  • Sporadic: trisomies 7, 16, 17.
  • Familial: trisomy 7.
    • Chromosome 7 = location of MET gene.

Note:

  • Not used for diagnosis.[23]

Sign out

Kidney Tumour, Left, Partial Nephrectomy: 
- PAPILLARY RENAL CELL CARCINOMA. 
-- Surgical margins NEGATIVE. 
-- ISUP nucleolar (Fuhrman) Grade 3. 
-- TNM stage: pT1a pNx. 
-- Please see tumour summary.

Block letters

KIDNEY, RIGHT, RADICAL NEPHRECTOMY:
- PAPILLARY RENAL CELL CARCINOMA, WHO/ISUP NUCLEOLAR GRADE 3, pT2a(2), pNx.
-- SURGICAL MARGINS NEGATIVE.
-- PLEASE SEE TUMOUR SUMMARY.
- RENAL PAPILLARY ADENOMAS.

Micro

The sections show a tumour in the kidney with fibrovascular cores (papillae) that focally contain macrophages. Psammoma bodies are present. Siderophages are present.

The papillae predominantly have a single layer of tumour cells and the cytoplasm of the tumour cells is predominantly clear.

Nucleoli are visible focally with the 10x objective (ISUP nucleolar grade 3).

A second tumour with the same morphology is present and measures 8 millimetres.

Multiple small lesions, like the largest tumour, less than 1.5 cm are present.

Oncocytic variant

KIDNEY, RIGHT, NEPHRECTOMY:
- PAPILLARY RENAL CELL CARCINOMA, ONCOCYTIC -- SEE COMMENT;
- WHO/ISUP GRADE 2;
- SURGICAL MARGINS NEGATIVE;
- PLEASE SEE TUMOUR SUMMARY.

COMMENT:
The oncocytic variant of papillary renal cell carcinoma (RCC) is uncommon and not widely
recognized as a subtype of papillary RCC.  The prognostic significance of the oncocytic 
cytoplasm is uncertain.[1]  The histomorphology in this case is compatible with a type 1 
papillary RCC.

1. Ann Diagn Pathol. 2006 Jun;10(3):133-9.

See also

References

  1. ↑ 1.0 1.1 1.2 Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 289. ISBN 978-0443066771.
  2. ↑ Klatte, T.; Pantuck, AJ.; Said, JW.; Seligson, DB.; Rao, NP.; LaRochelle, JC.; Shuch, B.; Zisman, A. et al. (Feb 2009). "Cytogenetic and molecular tumor profiling for type 1 and type 2 papillary renal cell carcinoma.". Clin Cancer Res 15 (4): 1162-9. doi:10.1158/1078-0432.CCR-08-1229. PMID 19228721.
  3. ↑ Fogo, Agnes B.; Kashgarian, Michael (2005). Diagnostic Atlas of Renal Pathology: A Companion to Brenner and Rector's The Kidney 7E (1st ed.). Saunders. pp. 438. ISBN 978-1416028710.
  4. ↑ Czene, K.; Hemminki, K. (Apr 2003). "Familial papillary renal cell tumors and subsequent cancers: a nationwide epidemiological study from Sweden.". J Urol 169 (4): 1271-5. doi:10.1097/01.ju.0000052373.36963.12. PMID 12629341.
  5. ↑ Wadt, KA.; Gerdes, AM.; Hansen, TV.; Toft, BG.; Friis-Hansen, L.; Andersen, MK. (Sep 2012). "Novel germline c-MET mutation in a family with hereditary papillary renal carcinoma.". Fam Cancer 11 (3): 535-7. doi:10.1007/s10689-012-9542-6. PMID 22717761.
  6. ↑ Siracusano, S.; Novara, G.; Antonelli, A.; Artibani, W.; Bertini, R.; Carini, M.; Carmignani, G.; Ciciliato, S. et al. (Dec 2012). "Prognostic role of tumour multifocality in renal cell carcinoma.". BJU Int 110 (11 Pt B): E443-8. doi:10.1111/j.1464-410X.2012.11121.x. PMID 22502873.
  7. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1017-8. ISBN 0-7216-0187-1.
  8. ↑ Granter SR, Perez-Atayde AR, Renshaw AA (October 1998). <303::AID-CNCR6>3.0.CO;2-7 "Cytologic analysis of papillary renal cell carcinoma". Cancer 84 (5): 303?8. PMID 9801205. http://dx.doi.org/10.1002/(SICI)1097-0142(19981025)84:5<303::AID-CNCR6>3.0.CO;2-7.
  9. ↑ 9.0 9.1 Moch, H.; Cubilla, AL.; Humphrey, PA.; Reuter, VE.; Ulbright, TM. (Feb 2016). "The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours.". Eur Urol. doi:10.1016/j.eururo.2016.02.029. PMID 26935559.
  10. ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 288. ISBN 978-0443066771.
  11. ↑ The International Agency for Research on Cancer (Author), J. Eble (Editor), J. Epstein (Editor), I. Sesterhenn (Editor), G. Sauter (Editor) (2004). Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs (IARC WHO Classification of Tumours) (1st ed.). Lyon: World Health Organization. pp. 28. ISBN 978-9283224150.
  12. ↑ Rogala J, Kojima F, Alaghehbandan R, Agaimy A, Martinek P, Ondic O, Ulamec M, Sperga M, Michalova K, Pivovarcikova K, Pitra T, Hora M, Ferak I, Marečková J, Michal M, Hes O (February 2020). "Papillary renal cell carcinoma with prominent spindle cell stroma - tumor mimicking mixed epithelial and stromal tumor of the kidney: Clinicopathologic, morphologic, immunohistochemical and molecular genetic analysis of 6 cases". Ann Diagn Pathol 44: 151441. doi:10.1016/j.anndiagpath.2019.151441. PMID 31862520.
  13. ↑ Al-Obaidy KI, Eble JN, Cheng L, Williamson SR, Sakr WA, Gupta N, Idrees MT, Grignon DJ (August 2019). "Papillary Renal Neoplasm With Reverse Polarity: A Morphologic, Immunohistochemical, and Molecular Study". Am J Surg Pathol 43 (8): 1099–1111. doi:10.1097/PAS.0000000000001288. PMID 31135486.
  14. ↑ Delahunt, B.; Eble, JN. (Jun 1997). "Papillary renal cell carcinoma: a clinicopathologic and immunohistochemical study of 105 tumors.". Mod Pathol 10 (6): 537-44. PMID 9195569.
  15. ↑ Srigley, JR.; Delahunt, B. (Jun 2009). "Uncommon and recently described renal carcinomas.". Mod Pathol 22 Suppl 2: S2-S23. doi:10.1038/modpathol.2009.70. PMID 19494850.
  16. ↑ Hes, O.; Brunelli, M.; Michal, M.; Cossu Rocca, P.; Hora, M.; Chilosi, M.; Mina, M.; Boudova, L. et al. (Jun 2006). "Oncocytic papillary renal cell carcinoma: a clinicopathologic, immunohistochemical, ultrastructural, and interphase cytogenetic study of 12 cases.". Ann Diagn Pathol 10 (3): 133-9. doi:10.1016/j.anndiagpath.2005.12.002. PMID 16730306.
  17. ↑ Han, G.; Yu, W.; Chu, J.; Liu, Y.; Jiang, Y.; Li, Y.; Zhang, W. (Jan 2017). "Oncocytic papillary renal cell carcinoma: A clinicopathological and genetic analysis and indolent clinical course in 14 cases.". Pathol Res Pract 213 (1): 1-6. doi:10.1016/j.prp.2016.04.009. PMID 27931799.
  18. ↑ http://surgpathcriteria.stanford.edu/kidney/papillary-renal-cell-carcinoma/differential-diagnosis.html. Accessed on: 6 May 2014.
  19. ↑ Saleeb RM, Brimo F, Farag M, Rompré-Brodeur A, Rotondo F, Beharry V, Wala S, Plant P, Downes MR, Pace K, Evans A, Bjarnason G, Bartlett JMS, Yousef GM (December 2017). "Toward Biological Subtyping of Papillary Renal Cell Carcinoma With Clinical Implications Through Histologic, Immunohistochemical, and Molecular Analysis". Am J Surg Pathol 41 (12): 1618–1629. doi:10.1097/PAS.0000000000000962. PMID 28984673.
  20. ↑ Ono, Y.; Ito, T.; Tsujino, S.; Aizawa, S.; Suzuki, M. (Jun 1997). "[A study of papillary renal cell carcinoma. Clinicopathological, immunohistochemical features and its typing].". Nihon Hinyokika Gakkai Zasshi 88 (6): 587-95. PMID 9234615.
  21. ↑ Watanabe, S.; Naganuma, H.; Shimizu, M.; Ota, S.; Murata, S.; Nihei, N.; Matsushima, J.; Mikami, S. et al. (2013). "Adult nephroblastoma with predominant epithelial component: a differential diagnostic candidate of papillary renal cell carcinoma and metanephric adenoma-report of three cases.". Case Rep Pathol 2013: 675875. doi:10.1155/2013/675875. PMID 24083046.
  22. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1016. ISBN 0-7216-0187-1.
  23. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 292. ISBN 978-0781765275.
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